A 20 year old lady presented with h/o sleeping 20 hours a day from 10 days. There was h/o right hemiparesis 2 years ago.
Her MRI brain showed signal changes in hypothalamus, periaqueductal grey and pontine tegmentum. 
Serum Aquaporin-4 antibody test (by immunofluorescence method) was done, and the result was POSITIVE.
This is one of the unusual presentation of a neurological disorder Neuromyelitis optica (NMO), which usually presents with optic neuritis and transverse myelitis.
Narcolepsy is a sleep disorder characterized by excessive daytime sleepiness.
Primary narcolepsy is caused by selective loss of hypocretin-1-producing neurons. HLA DQB1*0602 is found in more than 85% of people with primary narcolepsy.
Primary narcolepsy is caused by selective loss of hypocretin-1-producing neurons. HLA DQB1*0602 is found in more than 85% of people with primary narcolepsy.
Symptomatic narcolepsy occurs rarely with neurologic disorders such as tumor, stroke, or demyelinating disorders that directly damage hypocretin-1-producing neurons in the hypothalamus.
Narcolepsy has been reported in autoimmune demyelinating disorders - Multiple sclerosis and NMO.
Our patient had narcolepsy as the presenting symptom with signal changes in hypothalamus on MRI.
Our patient had narcolepsy as the presenting symptom with signal changes in hypothalamus on MRI.
Traditionally, NMO was considered as a disease of spinal cord and optic nerves. The discovery of aquaporin-4 IgG antibodies specific for NMO has broadened the clinical and neuroimaging spectrum of NMO with increasing recognition of nonopticospinal forms.
Symptomatic narcolepsy is one of the core clinical characteristics in the revised international consensus diagnostic criteria (2015) for NMO spectrum disorders.
Core clinical features of NMOSD:
1. Optic neuritis
2. Acute myelitis
3. Area postrema syndrome
4. Acute brainstem syndrome
5. Symptomatic narcolepsy (acute diencephalic syndrome)
6. Symptomatic cerebral syndrome with typical brain lesions.
1. Optic neuritis
2. Acute myelitis
3. Area postrema syndrome
4. Acute brainstem syndrome
5. Symptomatic narcolepsy (acute diencephalic syndrome)
6. Symptomatic cerebral syndrome with typical brain lesions.
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